C9orf72-generated poly-GR and poly-PR do not directly interfere with nucleocytoplasmic transport
العنوان: | C9orf72-generated poly-GR and poly-PR do not directly interfere with nucleocytoplasmic transport |
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المؤلفون: | Thomas Vercruysse, Dirk Daelemans, Joni Vanneste, Philip Van Damme, Adria Sicart, Ludo Van Den Bosch, Steven Boeynaems |
المصدر: | Scientific Reports BASE-Bielefeld Academic Search Engine Scientific Reports, Vol 9, Iss 1, Pp 1-10 (2019) |
بيانات النشر: | Springer Science and Business Media LLC, 2019. |
سنة النشر: | 2019 |
مصطلحات موضوعية: | STRESS, Induced Pluripotent Stem Cells, Active Transport, Cell Nucleus, lcsh:Medicine, C9ORF72, Receptors, Cytoplasmic and Nuclear, Karyopherins, Article, DIPEPTIDE-REPEAT PROTEINS, C9orf72, Cell Line, Tumor, GGGGCC REPEAT, Genes, Regulator, Humans, lcsh:Science, Receptor, Motor Neurons, Science & Technology, HEXANUCLEOTIDE REPEAT, DNA Repeat Expansion, Multidisciplinary, C9orf72 Protein, Chemistry, IMPORT, lcsh:R, Amyotrophic Lateral Sclerosis, Translation (biology), EXPANSION, Dipeptides, Cell biology, Multidisciplinary Sciences, Microscopy, Fluorescence, Cell culture, Nucleocytoplasmic Transport, Frontotemporal Dementia, Fatty Acids, Unsaturated, Science & Technology - Other Topics, lcsh:Q, TRANSLATION, Nuclear transport, NUCLEAR-PORE COMPLEXES, PHASE-SEPARATION, HeLa Cells |
الوصف: | Repeat expansions in the C9orf72 gene cause amyotrophic lateral sclerosis and frontotemporal dementia characterized by dipeptide-repeat protein (DPR) inclusions. The toxicity associated with two of these DPRs, poly-GR and poly-PR, has been associated with nucleocytoplasmic transport. To investigate the causal role of poly-GR or poly-PR on active nucleocytoplasmic transport, we measured nuclear import and export in poly-GR or poly-PR expressing Hela cells, neuronal-like SH-SY5Y cells and iPSC-derived motor neurons. Our data strongly indicate that poly-GR and poly-PR do not directly impede active nucleocytoplasmic transport. ispartof: SCIENTIFIC REPORTS vol:9 issue:1 ispartof: location:England status: published |
وصف الملف: | Electronic |
تدمد: | 2045-2322 |
الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::07f6e601fe585f091b8a0affd17ebc63Test https://doi.org/10.1038/s41598-019-52035-6Test |
حقوق: | OPEN |
رقم الانضمام: | edsair.doi.dedup.....07f6e601fe585f091b8a0affd17ebc63 |
قاعدة البيانات: | OpenAIRE |
تدمد: | 20452322 |
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